Tuberous sclerosis-associated pulmonary lymphangioleiomyomatosis: The role of pulmonary rehabilitation - A case report

Abstract

Lymphangioleiomyomatosis (LAM) is a rare lung disease that primarily affects women. A patient with a medical history of Tuberous Sclerosis-Associated Lymphangioleiomyomatosis (TSC-LAM), a prior thyroidectomy for thyroid cancer, chronic hypertension, and a previous pulmonary thromboembolism was admitted to the hospital. Following the stabilization of her clinical condition, diaphragmatic exercises and incentive spirometers were implemented. This intervention significantly enhanced her respiratory status, prevented the need for invasive mechanical ventilation, and expediting pulmonary functional recovery. While further studies are needed, pulmonary rehabilitation has the potential to influence the clinical course of TSC-LAM patients in the ICU by improving respiratory capacity and reducing hospitalization time.

Keywords

• Lymphangioleiomyomatosis
• Pulmonary rehabilitation
• TSC-LAM
• S-LAM
• Klebsiella pneumonia